CARDIOVASCULAR JOURNAL OF AFRICA • Volume 34, No 1, January–April 2023 44 AFRICA The optimal diagnosis and treatment of intravenous leimyomatosis Hai-Yuan Liu, Jin-Guo Xu, Cheng-Xin Zhang Abstract Intravenous leiomyomatosis (IVL) is a rare form of gynaecological-uterine leiomyoma. Clinically, the diagnosis and treatment are more difficult and challenging due to occult symptoms and clinical presentations, which can be similar to other common diseases. In this report, comprehensive management of a case of IVL is reported and discussed, with the aim of sharing our academic and clinical experience to improve the medical management of IVL. Keywords: intravenous leimymatosis, diagnosis, treatment, cardiopulmonary bypass Submitted 10/4/21; accepted 20/12/21 Published online 1/7/22 Cardiovasc J Afr 2023; 34: 44–47 www.cvja.co.za DOI: 10.5830/CVJA-2022-002 Intravenous leiomyomatosis (IVL), although classified as a benign tumour, is characterised as inward growth towards the vascular wall, with some of the pathological behaviours of a malignant tumour, including metastasis and invasion. Growth and proliferation of the IVL are accelerated and diffuse in the uterus and its surrounding vascular system. This may involve the iliac vein, inferior vena cava or even the right heart system, leading to serious adverse events, including heart failure and pulmonary embolism and causing great risk to the life of patients.1 Early clinical symptoms of IVL are not significant and diagnosis is often missed due to its occult progress. Later, the surgical risk will increase dramatically following significant symptoms of heart failure. We reviewed the surgical intervention of a typical case of IVL involving the right atrium, with the aim of sharing our surgical experience of IVL. Case report The 50-year-old female patient was admitted with symptoms of chest distress of 15 days’ duration. A previous medical institute had diagnosed an IVL from colour Doppler ultrasound, with a description of diffuse, irregular filling defects found within the right atrium, inferior vena cava, left renal vein and left ovarian vein. The patient received further imaging tests in our hospital. Abdominal ultrasound showed diffuse solid masses with low solid echo within the uterus, and the tumour body extended from the left para-uterine vein to the right atrium through the left ovarian vein, left renal vein and inferior vena cava. The size of the mass within the right atrium was 4 × 3 cm, and during diastole, the mass would pass the orifice of the tricuspid valve, leading to mild pulmonary hypertension on echocardiography. Under computerised tomography (CT) scanning, huge masses were discovered within the abdominal and pelvic cavity, involving the left ovarian vein, renal vein, inferior vena cava and right atrium. Similarly, magnetic resonance imaging (MRI) detected abnormal signals within the abdominal and pelvic cavity, right atrium, inferior vena cava, left renal vein and left ovarian vein (Fig. 1). A diagnosis of IVL was supported by the results of these imaging tests. After evaluation of the tests, the patient was referred for surgical treatment of the IVL. Transoesophageal echocardiography was used for observation and guidance during the procedure. A combined thoracic–abdominal incision was made. First, the uterus was detected through a median abdominal incision. Morphologically, the swollen uterus was irregular, with a smooth serosal layer. The diameter of the primary IVL was appropriately 17 cm. The tumour body within the left ovarian vein was found and removed. The remaining uterus was also removed and the left renal vein was completely exposed. Second, after systematic heparinisation, the tumour body within the right atrium was detected through a median sternal incision. Simultaneously, assisted cardiopulmonary bypass was established via implanted catheters in the aorta, femoral artery and superior vena cava at a constant temperature without perfusion of cardioplegia. The extended body of the IVL, appropriately 4 × 3 cm, was found at the orifice of the inferior vena cava, connected to the remaining tumour within the inferior vena cava. When separation of the tumour body within the renal vein was completed, the remaining extra-uterine tumour of the IVL was fully removed via the orifice of the inferior vena cava (Fig. 2). Finally, the patient was weaned from cardiopulmonary bypass and protamine was used to neutralise the heparin. After total haemostasis, the thoracic–abdominal incision was carefully closed. The vital signs of this patient were stable and she was monitored continuously during the entire procedure (Fig. 3). Based on the findings of postoperative pathology, under microscopic examination, the tumour was diagnosed as a spindle cell tumour. Also, the immunohistochemistry showed KI-67(+0.2%), P53(–), VIM(–), SMA(+), DES(+), CD10(–), CD34(–) and S100(–). Final diagnosis of the IVL was therefore confirmed via a pathology test. The patient was successfully discharged after some supportive and regular medical interventions (Fig. 4). Department of Cardiovascular Surgery, The First Affiliated Hospital of Anhui Medical University, Anhui, China Hai-Yuan Liu, MD Jin-Guo Xu, MD Cheng-Xin Zhang, MD, zcxayd@163.com
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